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Diseases of the Respiratory System

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1 Diseases of the Respiratory System
Chronic Obstructive Pulmonary Diseases (COPD) Prof. Ammar C. Al-Rikabi Dr. Maha Arafah Respiratory block 2019 Pathology Lecture 2

2 Objectives: Give introduction for diffuse lung disease
Compare and contrast the major clinical and functional differences between predominant chronic bronchitis versus predominant emphysema in patients with COPD Define Bronchiectasis, its causes, presentation, morphology and significant.

3 Obstructive Lung Diseases
(diffuse) Bronchial Asthma 2) Chronic obstructive pulmonary disease (COPD) Cigarette smoking is the principle cause 10% of population above 45 year has airflow obstruction They are of two types: a) Chronic bronchitis b) Emphysema 3) Bronchiectasis Common symptoms in lung disease Dyspnea: difficulty with breathing Cough Hemoptysis Robbins Basic Pathology Table 13.1 page 498

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5 Diseases of the Respiratory System
Emphysema Objectives: Define emphysema. Describe the gross and microscopic changes in emphysema. Discuss the typical clinical presentation and causes of death. Describe the most likely mechanism of emphysema (the protease-antiprotease mechanism). Describe the pathophysiologic mechanisms of emphysema

6 Emphysema Emphysema abnormal permanent enlargement of all or part of the respiratory unit accompanied by destruction of their walls Associated with loss of elastic recoil and support of small airways leading to tendency to collapse with obstruction

7 Classification of emphysema
Diseases of Lung Centriacinar: in heavy smoker, severe in upper lobes 2. Alpha 1 antitrypsin deficiency: Commonly affect both lower lobes at lower zones 3.Distal acinar /paraseptal: adjacent to areas of fibrosis or atelectasis More severe in the upper half of the lungs, young adult, unknown cause, spontaneous peumothorax 4. Irregular Associated with scarring, e.g. in TB Asymptomatic Classification of emphysema

8 Diseases of Lung Emphysema: morphology
Generalized emphysema. (a) Normal distal lung acinus. (b) Centriacinar emphysema. (c) Centriacinar emphysema. (d) Panacinar emphysema. (e) Panacinar emphysema (Gough-Wentworth section).

9 Diseases of Lung Emphysema

10 Bullous emphysema with large apical and subpleural bullae

11 Distal acinar /paraseptal:
forming multiple cyst-like structures with spontaneous pneumothorax.

12 Emphysema: Pathogenesis

13 Emphysema: Pathogenesis
Pathogenesis of Emphysema

14 destruction of alveolar walls
Emphysema: morphology Destruction of some alveolar septi Enlarged air spaces/alveolar spaces. Paraseptal emphysema, microscopic destruction of alveolar walls

15 Emphysema: morphology
Enlarged air spaces Destruction of some alveolar septi

16 Emphysema:- Clinical features: Dyspnea
Pursed lip expiration is a common maneuver adopted by patients with severe chronic obstructive pulmonary disease including emphysema. The patient starts to breathe out closed or nearly closed lips to keep the intrabronchial pressure high and prevent collapse of the bronchial wall and expiratory obstruction. Later in expiration the lips are blown forwards and open, often with a grunt (“fish-mouth breathing). Patient is sitting forward in a hunched- over position

17 Barrel-shaped chest in a patient with emphysema.
The hyperinflation result from air-trapping with inflammatory changes hypersecretion of viscid contraction in the small airways. Note the associated indrawing of the intercostal muscles. Similar changes are seen in patients with chronic bronchitis and asthma.

18 Right-sided heart failure (Cor pulmonale)
Emphysema: Complications Pneumothorax Pulmonary hypertension (due to destruction of small capillaries in alveolar wall and hypoxia lead to pulmonary vascular spasm) Right-sided heart failure (Cor pulmonale) Death from emphysema is related to: Pulmonary failure with respiratory acidosis, hypoxia and coma or due to pulmonary hypertension.

19 Emphysema: Types Clinical features Complications Centriacinar: Smoking
Emphysema: SUMMARY Emphysema: Dilated air spaces beyond respiratory bronchioles due to destruction of alveolar septa Centriacinar: Smoking Panacinar: deficiency of α1 AT Paraseptal: Occurs adjacent to areas of fibrosis or atelectasis. Irregular: scar Types Cough and wheezing. Dyspnea Weight loss Pulmonary function tests reveal low FEV1 Pink puffers. Respiratory acidosis Clinical features Pneumothorax Pulmonary hypertension. Right-sided heart failure (Cor pulmonale) Pulmonary failure with respiratory acidosis, hypoxia and coma. Complications

20 Diseases of the Respiratory System
Chronic Bronchitis Objectives: Define chronic bronchitis. Describe the causes, pathogenesis and the morphology of chronic bronchitis. Describe the mechanism of airway obstruction in a patient with chronic bronchitis. Understand that when severe obstruction is present in chronic bronchitis, significant emphysema is nearly always present

21 Causes Cigarette smoking is the most important risk factor
Chronic Bronchitis Chronic Bronchitis: defined as persistent productive cough for at least 3 consecutive months in at least 2 consecutive years Causes Cigarette smoking is the most important risk factor Air pollutants Cystic fibrosis

22 Chronic Bronchitis: morphology

23 Chronic Bronchitis: morphology

24 Chronic Bronchitis: morphology
Chronic bronchitis. In chronic bronchitis the main abnormality is secretion of abnormal amounts of mucus, causing plugging of the airway lumen (P)

25 Clinical features and compilcations
Chronic Bronchitis Clinical features and compilcations Persistent reproductive cough Dyspnea on exertion Hypercapnia, hypoxemia, cyanosis (blue bloaters) Emphysema Cor pulmonale Death due to further impairment of respiratory functions after superimposed acute baterial infections.

26 Causes Features Complications
Chronic Bronchitis Definition: Persistent productive cough (with sputum) for at least 3 months in at least 2 consecutive years Cigarette smoking is the most important risk factor; air pollutants also contribute Causes enlargement of mucous-secreting glands, goblet cell hyperplasia, chronic inflammation, and bronchiolar wall inflammation and fibrosis. Features Persistent reproductive cough, dyspnea on exertion, hypercapnia, hypoxemia, cyanosis, cor pulmonale with edema (blue bloater) Death may result from further impairment of respiratory function due to superimposed acute infections. Complications

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28 Bronchiectasis Objectives: Definition Causes Presentation
morphology and significant

29 Bronchiectasis Bronchiectasis is permenant dilatation of bronchi with destruction of their walls. It is a result of chronic inflammation associated with an inability to clear mucoid secretions.

30 Bronchial obstruction
us Causes of bronchiectasis Bronchial obstruction Congenital or hereditary conditions Chronic or severe infection / necrotizing pneumonia Localized: - tumor, foreign bodies or mucous impaction Generalized: - bronchial asthma - chronic bronchitis - Congenital bronchiectasis - Cystic fibrosis. - Intralobar sequestration of the lung. - Immunodeficiency status. - Immotile cilia and kartagner syndrome Caused by TB, staphylococci or mixed infection

31 Diseases of Lung Cilial dysmotility syndrome. Electron micrograph of cilia from a person with recurrent chest infections since childhood. The outer dynein arms are absent and there are abnormal single microtubules (M), which prevent normal motility.

32 Bronchiectasis, chest radiograph
Presentation of Bronchiectasis Severe, persistent cough associated with expectoration of mucopurulent, sometimes bad smell sputum.  Other common symptoms include dyspnea, rhinosinusitis, and hemoptysis. Bronchiectasis, chest radiograph

33 Bronchiectasis, gross

34 Diseases of Lung Bronchiectasis
Dilatation of bronchi with destruction of bronchial walls

35 Diseases of Lung Bronchiectasis. This is a lower lobe of lung surgically resected for bronchiectasis.

36 Bronchiectasis, micropscopic

37 Bronchiectasis Complications
Lung Abscess Bronchiectasis Complications Obstructive pulmonary function (hypoxemia, hypercapnia, pulmonary hypertension, and cor pulmonale) Lung Abscess Rare complications: include Metastatic brain (cerebral) abscess Amyloidosis. Brain (cerebral) abscess

38 Bronchiectasis: Infection/ Necrotizing pneumonia Obstruction
Dilatation and destruction of bronchi and bronchioles secondary to chronic inflammation and obstruction Bronchiectasis: Infection/ Necrotizing pneumonia Obstruction Congenital (Cystic fibrosis, Kartagener’s Syndrome) Causes Sever persistent cough with sputum (mucopurulent sputum) sometime with blood. Clubbing of fingers. Clinical features If sever, obstructive pulmonary function Lung Abscess Rare complications: metastatic brain(cerebral) abscess and amyloidosis. Complications

39 Diseases of Lung Key Facts Chronic obstructive pulmonary disease
. Definition: a disease state characterized by airflow limitations that are not fully reversible. The airflow limitation is usually both progressive and associated with an abnormal inflammatory response of the lungs to noxious particles or gases. . Cigarette smoking remains the most important cause of COPD. Other risks are recurrent chest viral infections in childhood, atopy, asthma, and occupational exposure to dusts (especially mining). . Respiratory bronchiolitis is one of the earliest lesions seen in smokers. . Chronic bronchitic airways show mucous hypersecretion with mucous gland hyperplasia. . Chronic bronchitis and bronchiolitis cause airway narrowing. . Emphysema causes loss of elastic recoil in lungs and contributes to functional airways obstruction. . Generalized emphysema is defined as permanent dilatation of any part of the respiratory acinus, with destruction of tissue in the absence of scarring. . There are two patterns of generalized emphysema: centrilobular and panacinar. . Many patients with COPD have a reversible component to functional airways obstruction. . Pulmonary hypertension and right-sided heart failure are common in long-standing chronic obstructive pulmonary disease. . Acute deterioration in COPD is usually caused by viral or bacterial infection.

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